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Article

Mutation Status and Epithelial Differentiation Stratify Recurrence Risk in Chordoid Meningioma—A Multicenter Study with High Prognostic Relevance

1
Department of Pathology, Louisiana State University, Shreveport, LA 71103, USA
2
Feist-Weiller Cancer Center, Shreveport, LA 71103, USA
3
NeuroMarkers Professional Limited Liability Company, Houston, TX 77025, USA
4
Department of Neurosurgery, Rutgers University, Camden, NJ 08901, USA
5
Department of Pathology, Vanderbilt University Medical Center, Nashville, TN 37232, USA
6
Department of Pathology and Cell Biology, Columbia University, New York, NY 10032, USA
7
Department of Pathology, the University of Texas Southwestern Medical Center, Dallas, TX 75390, USA
8
Department of Pathology and Laboratory Medicine and Neurosurgery, Medical University of South Carolina and Hollings Cancer Center, Charleston, SC 29425, USA
*
Author to whom correspondence should be addressed.
These authors contributed equally.
Cancers 2020, 12(1), 225; https://doi.org/10.3390/cancers12010225
Received: 9 November 2019 / Revised: 13 January 2020 / Accepted: 14 January 2020 / Published: 17 January 2020
Chordoid meningioma is a rare WHO grade II histologic variant. Its molecular alterations or their impact on patient risk stratification have not been fully explored. We performed a multicenter, clinical, histological, and genomic analysis of chordoid meningiomas from 30 patients (34 tumors), representing the largest integrated study to date. By NHERF1 microlumen immunohistochemical detection, three epithelial differentiation (ED) groups emerged: #1/fibroblastic-like, #2/epithelial-poorly-differentiated and #3/epithelial-well-differentiated. These ED groups correlated with tumor location and genetic profiling, with NF2 and chromatin remodeling gene mutations clustering in ED group #2, and TRAF7 mutations segregating in ED group #3. Mutations in LRP1B were found in the largest number of cases (36%) across ED groups #2 and #3. Pathogenic ATM and VHL germline mutations occurred in ED group #3 patients, conferring an aggressive or benign course, respectively. The recurrence rate significantly correlated with mutations in NF2, as single gene, and with mutations in chromatin remodeling and DNA damage response genes, as groups. The recurrence rate was very high in ED group #2, moderate in ED group #3, and absent in ED group #1. This study proposes guidelines for tumor recurrence risk stratification and practical considerations for patient management. View Full-Text
Keywords: chordoid meningioma; epithelial differentiation; NHERF1/EBP50; NF2; TRAF7; ATM; chromatin remodeling genes chordoid meningioma; epithelial differentiation; NHERF1/EBP50; NF2; TRAF7; ATM; chromatin remodeling genes
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MDPI and ACS Style

Georgescu, M.-M.; Nanda, A.; Li, Y.; Mobley, B.C.; Faust, P.L.; Raisanen, J.M.; Olar, A. Mutation Status and Epithelial Differentiation Stratify Recurrence Risk in Chordoid Meningioma—A Multicenter Study with High Prognostic Relevance. Cancers 2020, 12, 225. https://doi.org/10.3390/cancers12010225

AMA Style

Georgescu M-M, Nanda A, Li Y, Mobley BC, Faust PL, Raisanen JM, Olar A. Mutation Status and Epithelial Differentiation Stratify Recurrence Risk in Chordoid Meningioma—A Multicenter Study with High Prognostic Relevance. Cancers. 2020; 12(1):225. https://doi.org/10.3390/cancers12010225

Chicago/Turabian Style

Georgescu, Maria-Magdalena, Anil Nanda, Yan Li, Bret C. Mobley, Phyllis L. Faust, Jack M. Raisanen, and Adriana Olar. 2020. "Mutation Status and Epithelial Differentiation Stratify Recurrence Risk in Chordoid Meningioma—A Multicenter Study with High Prognostic Relevance" Cancers 12, no. 1: 225. https://doi.org/10.3390/cancers12010225

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